Kidney dysplasia is when one or both kidneys don’t develop properly during fetal development. Healthcare providers usually diagnose kidney dysplasia during pregnancy or infancy. They aren’t sure what causes most cases. Mild cases may not need treatment. Severe cases may be fatal.
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Kidney dysplasia is a condition in which one or both kidneys don’t develop the way they should while a fetus grows in the uterus. It’s one of several types of congenital urinary anomalies. Another name for congenital urinary anomalies is congenital anomalies of the kidney and urinary tract (CAKUT). If your child has kidney dysplasia (KID-nee dis-PLAY-zhuh), the normal structures in the kidney:
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Your child’s kidneys are also usually smaller than normal (renal hypodysplasia). They also may contain fluid-filled sacs (kidney cysts), which can make them appear larger than they should.
Throughout the world, healthcare experts estimate that between 2 and 4 out of every 1,000 babies have kidney dysplasia at birth.
Other names for kidney dysplasia include:
If kidney dysplasia affects one of your kidneys, healthcare providers call it unilateral kidney dysplasia. Bilateral kidney dysplasia affects both kidneys.
Kidney hypodysplasia refers to smaller kidneys that result from kidney dysplasia, have fewer nephrons and may contain kidney cysts.
Kidney dysplasia itself usually doesn’t have symptoms. But if problems develop in the urinary system along with kidney dysplasia, you may develop:
Kidney dysplasia that leads to severe, untreated kidney failure may lead to a buildup of waste products in your blood (uremia). If uremia develops, you may experience some of the following symptoms:
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In most cases, experts aren’t sure what causes kidney dysplasia. But there are two main categories of known causes: genetic and environmental.
With genetic causes of kidney dysplasia, it can be a problem with how the genes tell the kidneys to form during fetal development. Experts have learned a lot in the past few years about how genetic variations can lead to kidney dysplasia and other forms of CAKUT. The exact affected genes can determine whether kidney dysplasia develops by itself or there’s an association with other developmental anomalies.
Environmental causes of kidney dysplasia occur when you take certain medications or other drugs during pregnancy. These may include:
It’s a good idea to talk to a healthcare provider about medications you’re taking if you’re pregnant or planning to become pregnant.
Anyone can develop kidney dysplasia. But a baby’s risk of having kidney dysplasia is higher if:
Kidney disease also affects males slightly more than females — 1.3 males to every 1 female.
If kidney dysplasia is severe in both kidneys, it may cause chronic kidney disease (CKD) at birth that can progress to kidney failure. If kidney dysplasia is only present in one kidney, your baby may never develop complications from it. If kidney dysplasia is due to a genetic cause, it may associate with other problems in the development of your baby’s urinary tract (ureters, bladder) or other organs.
It depends. It’s about twice as common for kidney dysplasia to affect only one kidney. In these cases, the remaining kidney will get bigger and stronger to “make up” for the dysplastic kidney. It may never affect your child’s health. But if kidney dysplasia affects both kidneys, your child has a higher chance of developing chronic kidney disease.
With severe kidney dysplasia in both kidneys, kidney function may be very poor at birth. If kidney dysplasia is severe in one kidney and mild in the other, kidney function may not decrease until later in life. Decreased kidney function at birth increases the chance of developing kidney failure early in life.
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Kidney dysplasia may be the only problem that develops. Or it could be part of a larger problem during fetal development. In such cases, other congenital conditions may be present in the fetus or at birth.
Healthcare providers most often diagnose kidney dysplasia during a prenatal ultrasound. They’ll confirm their diagnosis by performing an ultrasound on your child after birth.
Providers will also order kidney function tests to see how well your child’s kidneys work.
Once healthcare providers identify kidney dysplasia, they may refer your child to a genetics team, especially if they find other abnormalities. The team will review your child’s case and determine if genetic testing is indicated. They’ll talk to you about what genetic testing means and how to interpret the results.
Kidney dysplasia is a problem in which the kidneys don’t develop properly. So, there’s currently no therapy to treat or reverse kidney dysplasia. Treatments can only address the complications of kidney dysplasia — like UTIs, CKD and others. Your child may not need treatment if they have one dysplastic kidney and their other kidney is healthy.
More severe cases of kidney dysplasia may cause CKD. Treating CKD complications may include managing:
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Severe kidney dysplasia that causes kidney failure requires dialysis or a kidney transplant.
Schedule regular appointments with a pediatric nephrologist to monitor your child’s kidney health. Your child should begin seeing a pediatric nephrologist shortly after birth. How often they need to see the nephrologist depends on their kidney function and the presence of other complications.
During your appointments, you may wish to ask the following questions:
It depends. If your child has mild kidney dysplasia that only affects one kidney, they can grow and develop as expected with few or no problems. Their unaffected kidney will grow bigger and stronger to compensate for the affected kidney. But your child will need regular checkups with a kidney specialist (pediatric nephrologist) to ensure their unaffected kidney stays healthy.
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Fetuses with severe kidney dysplasia that affects both kidneys may not live long after birth. If they do, they’ll likely develop kidney failure and require dialysis or a kidney transplant. Kidney failure is fatal without dialysis or a transplant. Providers often identify these severe cases during pregnancy. They may refer you to a high-risk obstetrician (OB) specialist for more testing. Your team may involve a kidney specialist to talk about potential challenges or complications to expect at birth.
If your child has kidney dysplasia in one kidney, you must work with their treatment team to keep their unaffected kidney as healthy as possible. This may include:
If your child has kidney dysplasia in both kidneys, they must work closely with a pediatric nephrologist to manage complications of CKD or kidney failure.
Currently, there’s no way to prevent kidney dysplasia.
Multicystic dysplastic kidney (MCDK) is a kidney that doesn’t function because of kidney dysplasia that largely replaces the kidney with cysts. The kidney only contains nonfunctioning tissue.
Learning that your child has a congenital condition is one of the biggest fears for most parents. You may feel frightened, overwhelmed or that you’re at fault. As you come to terms with a kidney dysplasia diagnosis, it’s important to give yourself space to learn about the condition and its severity. Healthcare providers understand your feelings. They’re here to support you and offer advice every step of the way.
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Cleveland Clinic's health articles are based on evidence-backed information and review by medical professionals to ensure accuracy, reliability, and up-to-date clinical standards.
Cleveland Clinic's health articles are based on evidence-backed information and review by medical professionals to ensure accuracy, reliability, and up-to-date clinical standards.
When your child has kidney problems, you’ll want the best care. Cleveland Clinic’s experts in kidney care for children are here to help.
